Pattern of systemic lupus erythematosus in Egyptian patients: the impact of disease activity on the quality of life.

INTRODUCTION
Systemic lupus erythematosus (SLE) afflicts young people disproportionately, often at a crucial time in their lives when they are trying to establish relationships, start families and launch careers. As a result, persons with SLE may experience a wide range of physical and psychosocial problems that are not always fully captured by descriptions of the disease's physiological consequences alone.


METHODS
In order to characterize the spectrum of the effects of SLE with regards to disease activity and its impact on the quality of life (QoL), a case control study involving 59 SLE Egyptian patients (mean age 28.6 years, 94.9% females) and 20 healthy controls was undertaken. Disease activity was measured by SLE Disease Activity Index (SLEDAI), and quality of life was measured by Short Form-36 health questionnaire (SF-36).


RESULTS
Mucocutaneous and hematological manifestations were present in most of the patients and arthralgia in half of them. All domains of SF-36 including general health, physical functions, physical limitations, energy/fatigue, emotional well-being, pain, social functions, and health changes were significantly lower in SLE patients compared to controls. Except for emotional limitations, all domains were correlated with disease activity and low in class IV-V lupus nephritis.


CONCLUSION
Physicians should focus on QoL and how to improve it; health education regarding the negative impact of disease activity on the patients should be given attention. The results of QoL studies help physicians to understand and provide better support to SLE patients beside rapid meticulous control of disease activity.


Introduction Results
Out of 59 SLE patients, 56 were females (94.9%) and 3 patients (5.1%) were males. The age ranged from 16 to 42 years with a mean of 28.6±6.6 years. The mean disease duration was 5.6±3.4 years. There were no differences between cases and controls for demographic characteristic items (Table 1).
For clinical and biochemical findings (Table 2), malar rash was presented in 91.5% of the patients, photosensitivity was presented in 83.1% and discoid rash was presented in 20.3%. Eighty five percent (85%) of the patients were presented with mucosal ulcers. Arthritis was present in 52.5% of the SLE patients, while serositis (cardiopulmonary involvements) was present in 44.1% of them. 10.2% of the patients had had persistent proteinuria >0.5 grams/day or greater than 3+, 23.7% had cellular casts, and 54.2% had both proteinuria and urinary casts. About 51% of the patients had anemia, 55.9% had leucopenia and 33.9% had thrombocytopenia. 73% had elevated DNA and 6.8% had positive both anti-DNA and anti-phospholipids Abs.
According to SLEDAI score 51% of the SLE population in our study had mild activity and 27.2% had moderate to severe activity. No activity was present in 13%, while 16% had from moderate to severe activity.
Regarding renal pathology classification of lupus nephritis, 12% of the patients had class I, 5.1% had class II, 57.6% had class III, 20.3% class IV and 5.1% had class V. Table 3 shows the main domains of QoL measured by SF-36 in SLE patients compared to control. All domains of SF-36 including general health, physical functions, physical limitations, energy/fatigue, emotional well-being, pain, social functions, and health changes were lower in the SLE patients compared with control except emotional limitations. Table 4 shows that all domains of SF-36 including general health, physical functions, physical limitations, emotional limitations, energy/fatigue, emotional well-being, pain, social functions, and health changes were negatively correlated with grades of disease activity as detected by SLEDAI. Table 5 shows the mean score of each domains of QoL measured by SF-36 according to renal pathology classification of lupus nephritis in SLE patients. All domains of SF-36 including general health, emotional limitations, physical limitations, energy/fatigue, emotional well-being, pain, social functions, and health changes were lower in class IV-V the SLE patients compared with other classes except physical functions. Table 6 shows the correlation between QoL score measured by SF-36 and activity score measured by SLEDAI in SLE patients. The correlation was assessed through Pearson correlation. There are significant correlations between general health, pain and social functions domains in QoL score and activity score measured by SLEDAI in SLE patients.

Discussion
In the evaluation of patients with SLE it is important to measure not only disease activity (which is potentially reversible with treatment) and damage (which is permanent and can be due to the disease or treatment) but also the patients' perspective. This is because the disease is likely to have a significant impact on the physical, social and psychological aspects of the patient health and QoL [16].
The present clinical and biochemical data had revealed that, mucocutaneous and hematological manifestations were present in most of the . These variations may be due to different sample sizes, different patients' ages, variable diseases durations, unreported recent or mild cases, and seasonal, regional or racial variations.
Traditionally, SLE is a disease with great impact on all aspects of health status. QoL is increasingly being recognized as an important aspect of chronic diseases. Its measurement has traditionally relied on the use of generic or disease-specific questionnaires. Generic questionnaires were developed for general use and may be used in a variety of diseases and populations. They allow for comparison with other groups and conditions and allow measurement of dysfunction for individuals experiencing more than one condition. Therefore, general health instruments have been shown to be valid for measuring QoL in SLE patients. In contrast, disease-specific questionnaires are designed to measure outcomes in a specific disease [23]. Only recently have disease-specific instruments been developed for use in SLE and these are not yet widely used [16].
In our study, we observed a progressive decrease in all SF-36 scores. These progressive changes in HRQoL could be due to several factors, such as SLE progression along the years, continuously coping with a chronic illness, and practical management items that may be required (frequent medical visits, laboratory examinations, etc.). Other possible explanations are that, most of our SLE patients were young adults females, and in ages at which physical, psychological and social stability had not yet been reached. The disease started at a crucial time in their lives when they were trying to establish relationships, start families and launch careers. As a result, patients experienced a wide range of physical, psychological and social problems.
In addition, all domains of SF-36 including general health, physical functions, physical limitations, emotional limitations, energy/fatigue, emotional well-being, pain, social functions, and health changes were correlated with disease activity as detected by SLEDAI. In addition, we reported that the QoL of SLE patients as measured by SF-36 was poorer than healthy control in all domains except emotional limitations. Both physical and mental component summary scores of the SF-36 were reduced in our studied patients compared with controls. In SLE great variability in all the subscales was observed. Significant correlations between physical and mental components and different subscales were observed in patients but not in controls. In SLE, HRQoL tended to worsen with age. Higher disease activity and damage were associated with significantly lower HRQoL and worsening of SLE leads to a further decline.

Conclusion
Most of SLE studied patients were characterized predominantly by mucocutaneous and hematological manifestations. The QoL of SLE patients with renal involvement as measured by SF-36 was poorer than healthy control in all domains except emotional limitations. There are significant negative correlations between general health, pain and social functions domains in QoL score and activity score measured by SLEDAI in SLE patients.
Physicians should focus on QoL and how to improve it in relation to bio-psychosocial approach, (health education regarding the negative impact of disease activity on the patients is required). The results of quality of life studies help physicians understand and give better support to SLE patients beside rapid meticulous control of disease activity. Further researches on the factors affecting QoL in SLE patients, including drugs are required.